Neuropediatrics, Table of Contents Neuropediatrics 1999; 30(3): 146-148DOI: 10.1055/s-2007-973480 Short communications © Hippokrates Verlag GmbH Stuttgart Reduced Heart Rate Variability in Patients Affected with Rett Syndrome. A Possible Explanation for Sudden Death Francesca Guideri1 , M. Acampa1 , C. Hayek2 , M. Zappella2 , T. Di Perri1 1Institute of Clinical Medicine, School of Medicine, University of Siena, Siena, Italy 2Department of Child Neurology and Psychiatry, University of Siena, Siena, Italy Recommend Article Abstract PDF Download Buy Article Abstract Incidence of sudden death in Rett syndrome is greater than that of the general population and cardiac electrical instability is a prime suspect cause. Our study shows that girls with Rett syndrome had significantly lower heart rate variability (marker of autonomic disarray) and longer corrected QT intervals compared with an age-matched group of healthy girls. These abnormalities increased with advancing stages of the syndrome. These findings suggest a possible role of cardiac dysfunction in the sudden death associated with Rett syndrome. Key words Rett syndrome - Heart rate variability - Sudden death - QTc PDF (375 kb)
